TY - JOUR
T1 - Gut microbiota–host interactions in inborn errors of immunity
AU - Castagnoli, Riccardo
AU - Pala, Francesca
AU - Bosticardo, Marita
AU - Licari, Amelia
AU - Delmonte, Ottavia M.
AU - Villa, Anna
AU - Marseglia, Gian Luigi
AU - Notarangelo, Luigi Daniele
N1 - Funding Information:
This work was partially supported by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, and by the Bench to Bedside grant ?RAG deficiency: From pathophysiology to precise gene correction? (to L.D.N.). The article processing charges have been covered by the Pediatric Unit of the Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Pavia, Italy (to G.L.M.).
Publisher Copyright:
© 2021 by the authors. Licensee MDPI, Basel, Switzerland.
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2021/2/1
Y1 - 2021/2/1
N2 - Inborn errors of immunity (IEI) are a group of disorders that are mostly caused by genetic mutations affecting immune host defense and immune regulation. Although IEI present with a wide spectrum of clinical features, in about one third of them various degrees of gastrointestinal (GI) involvement have been described and for some IEI the GI manifestations represent the main and peculiar clinical feature. The microbiome plays critical roles in the education and function of the host’s innate and adaptive immune system, and imbalances in microbiota-immunity interactions can contribute to intestinal pathogenesis. Microbial dysbiosis combined to the impairment of immunosurveillance and immune dysfunction in IEI, may favor mucosal permeability and lead to inflammation. Here we review how immune homeostasis between commensals and the host is established in the gut, and how these mechanisms can be disrupted in the context of primary immunodeficiencies. Additionally, we highlight key aspects of the first studies on gut microbiome in patients affected by IEI and discuss how gut microbiome could be harnessed as a therapeutic approach in these diseases.
AB - Inborn errors of immunity (IEI) are a group of disorders that are mostly caused by genetic mutations affecting immune host defense and immune regulation. Although IEI present with a wide spectrum of clinical features, in about one third of them various degrees of gastrointestinal (GI) involvement have been described and for some IEI the GI manifestations represent the main and peculiar clinical feature. The microbiome plays critical roles in the education and function of the host’s innate and adaptive immune system, and imbalances in microbiota-immunity interactions can contribute to intestinal pathogenesis. Microbial dysbiosis combined to the impairment of immunosurveillance and immune dysfunction in IEI, may favor mucosal permeability and lead to inflammation. Here we review how immune homeostasis between commensals and the host is established in the gut, and how these mechanisms can be disrupted in the context of primary immunodeficiencies. Additionally, we highlight key aspects of the first studies on gut microbiome in patients affected by IEI and discuss how gut microbiome could be harnessed as a therapeutic approach in these diseases.
KW - Dysbiosis
KW - Gastrointestinal pathology
KW - Gut microbiota
KW - Immune dysregulation
KW - Inborn errors of immunity
KW - Primary immunodeficiencies
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U2 - 10.3390/ijms22031416
DO - 10.3390/ijms22031416
M3 - Review article
AN - SCOPUS:85100053614
VL - 22
SP - 1
EP - 19
JO - International Journal of Molecular Sciences
JF - International Journal of Molecular Sciences
SN - 1661-6596
IS - 3
M1 - 1416
ER -